E88.43

Disorders of mitochondrial tRNA synthetases

Billable FY2026 2025-10-01 → 2026-09-30

Exclusions 14 never code together

E88code

Excludes1

Excludes1

disorders of pyruvate metabolism (E74.4)E74.4
Kearns-Sayre syndrome (H49.81)H49.81
Leber's disease (H47.22)H47.22
Leigh's encephalopathy (G31.82)G31.82
Mitochondrial myopathy, NEC (G71.3)G71.3
Reye's syndrome (G93.7)G93.7

Excludes1

androgen insensitivity syndrome (E34.5-)E34.5-
congenital adrenal hyperplasia (E25.0)E25.0
hemolytic anemias attributable to enzyme disorders (D55.-)D55.-
Marfan syndrome (Q87.4-)Q87.4-
5-alpha-reductase deficiency (E29.1)E29.1

Excludes2

Excludes1

transitory endocrine and metabolic disorders specific to newborn (P70-P74)P70-P74

Excludes1 — never code together. The excluded condition and this one are mutually exclusive.

Excludes2 — not included here, but the patient may have both. Code both when documented.

Instructional notes 10

Inclusion terms

ARS2-related mitochondrial disorders
LBSL
Leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation
Leukoencephalopathy with thalamus - brainstem involvement - high lactate
LTBL
Mitochondrial aminoacyl-tRNA synthetase disorders

Code also

E88code

Use additional code

Notes

All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.E05.8 E07.0 E16-E31 E34.-

Index entries leading here 4

Disorder (of) — see also Disease
- metabolism NOS E88.9
  - mitochondrial E88.40
    - aminoacyl-tRNA synthetase E88.43
    - ARS2-related E88.43
    - tRNA synthetases E88.43
Leukoencephalopathy G93.49
- with
  - brainstem - spinal cord involvement - lactate elevation E88.43
  - thalamus - brainstem involvement - high lactate E88.43
LBSL (leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation) E88.43
LTBL (leukoencephalopathy with thalamus - brainstem involvement - high lactate) E88.43

Alongside this code 4 same parent — E88.4

Additional references

AHA Coding Clinic® Licence required

Official coding advice for this code. Published by the American Hospital Association. Not included in this installation.

CMS22HCC23 ESRD21HCC23 ESRD24HCC23 RX08HCC43

Presence is a property of the code. Untick a model to hide it everywhere.

Present FY2024–FY2027.